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Molecular mechanisms of neutrophil dysfunction in glycogen storage disease type Ib by Hyun Sik Jun, David A. Weinstein, Young Mok Lee, Brian C. Mansfield, and Janice Y. Chou Blood Volume 123(18):2843-2853 May 1, 2014 ©2014 by American Society of Hematology G6PT-deficient neutrophils of GSD-Ib patients with varying maturation states were dysfunctional. Hyun Sik Jun et al. Blood 2014;123:2843-2853 ©2014 by American Society of Hematology Analysis of 2-DG uptake, the expression of GLUT1 and HK, and levels of intracellular G6P, lactate, and ATP in G6PT-deficient neutrophils of GSD-Ib patients. Hyun Sik Jun et al. Blood 2014;123:2843-2853 ©2014 by American Society of Hematology Analysis of NADPH and the expression of NADPH oxidase in G6PT-deficient neutrophils of GSDIb patients. Hyun Sik Jun et al. Blood 2014;123:2843-2853 ©2014 by American Society of Hematology Analysis of levels of Hsp90, HIF-1α, and PPAR-γ in G6PT-deficient neutrophils and the effects of PPAR-γ antagonist/agonist on neutrophil function. Hyun Sik Jun et al. Blood 2014;123:2843-2853 ©2014 by American Society of Hematology Inhibition of neutrophil function by PPAR-γ is mediated via HIF-1α signaling. Hyun Sik Jun et al. Blood 2014;123:2843-2853 ©2014 by American Society of Hematology Proposed mechanisms that underlie neutrophil dysfunction in GSD-Ib. Hyun Sik Jun et al. Blood 2014;123:2843-2853 ©2014 by American Society of Hematology